Average Volume-assured Pressure Support as Rescue Therapy after CPAP Failure in Pediatric Obstructive Sleep Apnea: A Retrospective Case Series Study.

Background: Continuous positive airway pressure (CPAP) is frequently prescribed for patients with residual obstructive sleep apnea (OSA) following adenotonsillectomy. Objectives: The goal was to examine the efficacy of noninvasive ventilation with average volume-assured pressure support (AVAPS) as a potential option for children with failed CPAP titration. Methods: In a single-center retrospective study, we included children aged 1-17 years, with polysomnographically confirmed OSA who underwent AVAPS titration following failed CPAP titration. In addition to describing the clinical characteristics of the included patients, we compared polysomnographic parameters before and after AVAPS. Results: Nine patients met the inclusion criteria; out of them, 8 (89%) were males with an age range of 6.7 ± 3.9 years and a body mass index percentile of 81.0 ± 28.9. Reasons for failed CPAP titration were: 3 (33%) patients due to inability to control apnea-hypopnea index (AHI), 3 (33%) patients due to sleep-related hypoventilation, 2 (22%) patients due to treatment-emergent central sleep apnea, and 1 (11%) patient due to intolerance to CPAP. AVAPS resulted in a greater reduction in AHI than CPAP (reduction following CPAP = 24.6 ± 29.3, reduction following AVAPS = 42.5 ± 37.6, p = 0.008). All patients had resolution of the problems which caused CPAP failure. Conclusion: In this case a series of children with OSA and with failed CPAP titration, AVAPS resulted in a greater reduction in AHI compared with CPAP as well as resolution of the problems which caused CPAP failure.

Successful Treatment of Respiratory Failure in a Patient with Prader-Willi Syndrome with Noninvasive Ventilation with AVAPS.

Prader-Willi syndrome (PWS) is the most prevalent syndromic form of obesity, which starts during early childhood in the setting of hyperphagia. Due to the development of obesity, there is a high prevalence of obstructive sleep apnea (OSA) among these patients. This case report presents a patient with PWS with morbid obesity, severe OSA, and obesity hypoventilation syndrome admitted to the hospital for hypoxemic and hypercapnic respiratory failure. Noninvasive ventilation (NIV) with average volume-assured pressure support, a newer NIV modality, was used successfully to treat this patient, achieving major clinical and gas exchange improvement both during the hospitalization and long term after discharge.

A hospital-based multidimensional intervention for high risk pediatric patients with asthma.

OBJECTIVE: To determine the effects of a multi-dimensional intervention (consisting of education, recommendations for medical management, and short-term case management) provided by pulmonology nurse practitioners(NP) on inpatient and post-discharge outcomes for patients admitted with asthma exacerbations to a Pediatric Intensive Care Unit(PICU). METHODS: A retrospective cohort study was completed on subjects with an asthma exacerbation admitted to the PICU from 1 January 2015 to 31 December 2018. Records were reviewed for 12-months post-discharge. We compared inpatient and post-discharge outcomes for those who did vs. did not receive NP consultation. The primary outcome evaluated was optimization of discharge medications. Rates of follow up, repeat ED visits and hospitalizations were also reviewed. RESULTS: Two hundred and twenty two subjects met inclusion and exclusion criteria; of those, 101 (45.5%) patients received NP consultation and 121 (54.5%) had PICU management only. Patients with NP consultation were more likely to have controllers initiated (34.6% vs. 15%) or adjusted (55.5% vs. 33.3%) per asthma guidelines (p < 0.001). The consult group were more likely to have an asthma follow-up appointment made prior to discharge (99% vs. 45%, p < 0.001), and were more likely to attend the appointment (51% vs. 21%, p < 0.001). There were no significant differences between groups for ED visits or readmission for asthma 12-months post-discharge. CONCLUSIONS: Patients with NP consultation were more likely to have controllers started or adjusted per guidelines and were more likely to attend specialty follow-up appointments post-discharge. No impact was seen on ED visits or readmissions. Implementation of such a program may aid in optimizing asthma management and continuity of care post hospitalization.

Atraumatic Splenic Rupture Associated with Influenza A (H1N1) Pneumonia: Case Report and Review of the Literature.

Influenza virus infection may present with fever, chills, headache, myalgia, malaise, and respiratory symptoms, with a few cases developing into pneumonia, respiratory failure, and other organ damage. Very few cases of atraumatic splenic rupture associated with influenza infection have been reported. Atraumatic splenic rupture, while rare, is associated with high mortality. Here, we report the first case of atraumatic splenic rupture associated with influenza infection in the English literature and review the prior reported literature. The patient was diagnosed with influenza A (H1N1) pneumonia and subsequently developed hemorrhagic shock requiring emergency laparotomy and removal of the ruptured spleen.

Sleep-Disordered Breathing and Cardiovascular Disease in Children and Adolescents: A Scientific Statement From the American Heart Association.

Obstructive sleep apnea (OSA) is a known risk factor for cardiovascular disease in adults. It is associated with incident systemic hypertension, arrhythmia, stroke, coronary artery disease, and heart failure. OSA is common in children and adolescents, but there has been less focus on OSA as a primary risk factor for cardiovascular disease in children and adolescents. This scientific statement summarizes what is known regarding the impact of sleep-disordered breathing and, in particular, OSA on the cardiovascular health of children and adolescents. This statement highlights what is known regarding the impact of OSA on the risk for hypertension, arrhythmia, abnormal ventricular morphology, impaired ventricular contractility, and elevated right heart pressure among children and adolescents. This scientific statement also summarizes current best practices for the diagnosis and evaluation of cardiovascular disease-related complications of OSA in children and adolescents with sleep apnea and highlights potential future research in the area of sleep-disordered breathing and cardiovascular health during childhood and adolescence.

Granulomatous Pneumocystis jiroveci Pneumonia in an HIV-Positive Patient on Antiretroviral Therapy: A Diagnostic Challenge.

Human Immunodeficiency Virus (HIV)-related Opportunistic Infections (OI), including Pneumocystis jiroveci pneumonia (PCP), have become much less commonplace with anti-retroviral therapy (ART). Despite this, OIs are still common and it is important to remain vigilant for their presence and be aware of how ART and OI chemoprophylaxis may lead to atypical disease presentations. We present the case of a 51-year-old woman with HIV and CD4+ T helper lymphocytes cell count > 200 cells/ul on both ART and trimethoprim/sulfamethoxazole prophylaxis who presented with cavitating lung masses, mediastinal lymphadenopathy and pleural effusions. Negative bronchoalveolar lavage (BAL) and transbronchial biopsy (TBBx) prompted a second diagnostic procedure with a transthoracic core needle biopsy; the final diagnosis was granulomatous PCP. This case showcases a very rare presentation of PCP, with both large cavitating lung masses on imaging and granulomatous reaction on pathology, as well as the challenge of a potentially missed diagnosis with negative BAL and TBBx requiring transthoracic core needle biopsy for a final diagnosis.

Phenotypes of Bronchopulmonary Dysplasia in Adults.

Bronchopulmonary dysplasia (BPD), first described by Northway in 1967, is a process of neonatal lung injury that is most strongly associated with prematurity. The "old" form of the disease associated with the oxidative damage and volutrauma from perinatal mechanical ventilation has been increasingly supplanted by a "new" form resulting from interrupted growth of the lung at earlier stages of fetal development. Given the significant improvement in the survival of children with BPD since the 1980s, many more of these patients are living into adulthood and are being seen in adult pulmonary practices. In this review, we present three brief vignettes of patients from our practice to introduce three of the major patterns of disease seen in adult survivors of BPD, namely, asthma-like disease, obstructive lung disease, and pulmonary hypertension. Additional factors shown to affect the lives of adult BPD survivors are also discussed. Finally, we discuss insights into the process of transitioning these complex patients from pediatric to adult pulmonary practices. As survivors of BPD enter adulthood and continue to require specialty pulmonary care, awareness of the disease's varied manifestations and responses to treatment will become increasingly important.

Use of Noninvasive Ventilation with Volume-Assured Pressure Support to Avoid Tracheostomy in Severe Obstructive Sleep Apnea.

Obstructive sleep apnea (OSA) is a common disorder in children but can occasionally present with life-threatening hypoxemia. Obesity is a significant risk factor for poor outcomes of OSA treatment. Continuous positive airway pressure (CPAP) is indicated in children who are not candidates for or have an unsatisfactory response to adenotonsillectomy. Children acutely at risk for significant morbidity with other therapies are candidates for a tracheostomy. An eight-year-old patient with morbid obesity and severe OSA refractory to CPAP therapy was treated successfully with a novel noninvasive ventilation (NIV) mode with volume-assured pressure support (VAPS) and avoided tracheostomy.

Early childhood wheezers: identifying asthma in later life.

Wheeze in young children is common, and asthma is the most common noncommunicable disease in children. Prevalence studies of recurrent asthma-like symptoms in children under the age of 5 years have reported that one third of children in the US and Europe are affected, and rates and severity appear to be higher in developing countries. Over the last few decades, significant research efforts have focused on identification of risk factors and predictors of wheeze and on tools to identify which children who wheeze will progress to develop asthma. We reviewed the phenotypes of childhood wheezing, genetic risk factors, environmental factors, testing/predictive indices, and primary prevention. While it is generally agreed that a complex interaction of environmental exposure and genetic susceptibility contributes to the development of asthma, limitations in predictive tools and tests restrict our ability to provide families with guidance as to whether their child with wheeze will ultimately develop asthma. Additional research is needed to clarify childhood wheeze phenotypes, to develop tools to determine which children will develop asthma, and to determine how and when to intervene. If these areas can be addressed, it would help reduce this large burden on children, families, and society.

Effects of severe hypocapnia on expression of bax and bcl-2 proteins, DNA fragmentation, and membrane peroxidation products in cerebral cortical mitochondria of newborn piglets.

BACKGROUND: Hypocapnia occurs in the newborn infant inadvertently or as a therapeutic modality and may result in neuronal and mitochondrial alterations in the newborn brain. Since mitochondria regulate apoptosis, these alterations may initiate a cascade of reactions that lead to apoptotic cell death. OBJECTIVES: This study tests the hypothesis that hypocapnia results in increased expression of the pro-apoptotic protein Bax, fragmentation of DNA and membrane lipid peroxidation in cerebral cortical mitochondria (mt) of newborn piglets. METHODS: Studies were performed in three groups of anesthetized normoxic newborn piglets: hypocapnic (H, n = 5), ventilated at a PaCO(2) of 11-15 mm Hg; normocapnic (N, n = 5), ventilated at a PaCO(2) of 40 mm Hg; and corrected normocapnic (CN, n = 4), ventilated as H with CO(2) added to maintain normocapnia. Tissue ATP and phosphocreatine levels were determined. Mitochondrial membrane proteins were separated, transblotted and probed with antibodies to Bax and Bcl-2. Bands were detected by enhanced chemiluminescence and analyzed by imaging densitometry. mtDNA was isolated. Cell and mitochondrial membrane lipid peroxidation products were measured spectrofluorometrically. RESULTS: ATP and PCr concentrations were similar in the 3 groups. The ratio of Bax/Bcl-2 increased significantly in H compared to N and CN. mtDNA fragmentation was also significantly greater in H compared to N or CN. Membrane lipid peroxidation was higher in H than in N or CN; and in CN compared to N. CONCLUSIONS: The data demonstrate that severe hypocapnia results in increased Bax expression, DNA fragmentation, and membrane lipid peroxidation in mitochondria of cerebral cortical neurons of newborn piglets, and may result in apoptotic cell death.